22 March 2006 – Case of the Week #41
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We thank Dr. Anthony Martyniak, Beth Israel Deaconess Medical Center, Boston, Massachusetts (USA), for contributing this case. We invite you to contribute a Case of the Week by sending an email to NPernick@PathologyOutlines.com with microscopic images (any size, we will shrink if necessary) in JPG or GIF format, a short clinical history, your diagnosis and any other images (gross, immunostains, EM, etc.) that you have and that may be helpful or interesting. We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US) for your time after we send out the case. Please only send cases with a definitive diagnosis.
Case of the Week #41
Clinical history
A 72 year-old woman with a past medical history of hypertension and hypercholesterolemia presented with a several month history of multiple strokes. All causes of her strokes were ruled out, including infection and a hypercoagulable state. Numerous head CTs and MRIs revealed diffuse periventricular white matter change, thought to be chronic microvascular infarction. The patient’s mental status did not improve and a brain biopsy was ultimately performed.
The brain biopsy consisted of cortical gray and white matter with penetrating capillaries and arterioles containing large atypical cells (image1, image2). The cells were dyscohesive with irregular nuclear contours and one to several prominent nucleoli (image3). The cells were immunoreactive for leukocyte common antigen/CD45, CD20 (image4) and CD79a, and were negative for cytokeratins and HMB-45.
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Diagnosis:
Intravascular large B-cell lymphoma
Discussion
Intravascular lymphoma is a rare subtype of diffuse large B cell lymphoma, with intravascular growth in the skin, CNS, and other sites. The presenting signs and symptoms are often complex, and include mental status changes, as in this case, and rapidly progressive dementia. Patients may present with a mass lesion (Pathol Int 2004;54:231), a skin rash or fever of unknown origin.
Patients are typically elderly, with a median age of 71 years. Although chemotherapy may be effective (Ann Oncol 2004;15:1215), these tumors are often not diagnosed until autopsy, which typically shows involvement of most organs. In this case, the patient was 72 years old, and expired two weeks after the brain biopsy. A post-mortem examination revealed widespread disease, with involvement of the heart, lung, kidneys, adrenals, stomach, pancreas and spleen. No skin lesions were found.
Histologically, the tumor is composed of large centroblast-like lymphoid cells with prominent nucleoli within small vessel lumina, often capillaries. Mitotic figures are frequent, and there are often fibrin thrombi. Immunohistochemistry results are similar to diffuse large B cell lymphoma, with immunoreactivity for CD19, CD20, CD22 and CD79a. Tumors are usually bcl2 positive. Rarely, these tumors are T cell lymphomas with corresponding immunoreactivity.
Nat
Pernick, M.D.
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